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Sarah S Long
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December 2016: Journal of Pediatrics
Dilek Konukbay, Marco Gattorno, Dilek Yildiz, Joost Frenkel, Cengizhan Acikel, Betul Sozeri, Balahan Makay, Nuray Aktay Ayaz, Kenan Barut, Aysenur Kisaarslan, Yelda Bilginer, Dursun Karaman, Harun Peru, Dogan Simsek, Ozlem Aydog, Erbil Unsal, Zubeyde Gunduz, Berna Eren Fidanci, Isabelle Kone-Paut, Ozgur Kasapcopur, Angelo Ravelli, Seza Ozen, Erkan Demirkaya
OBJECTIVES: To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. METHODS: The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome...
September 2016: Clinical and Experimental Rheumatology
Antonio Vitale, Ida Orlando, Giuseppe Lopalco, Giacomo Emmi, Marco Cattalini, Bruno Frediani, Mauro Galeazzi, Florenzo Iannone, Donato Rigante, Luca Cantarini
OBJECTIVES: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non-Mendelian autoinflammatory disorder until now considered to be specifically limited to paediatric age. Recently, an increasing number of reports seems to suggest that PFAPA syndrome, diagnosed by the Marshall criteria revised by Thomas et al., can also affect adults. METHODS: The Marshall/Thomas criteria have been applied to 989 adult patients presenting for recurrent fever episodes: all patients enrolled were reviewed for demographic, clinical, and therapeutic data...
September 2016: Clinical and Experimental Rheumatology
Ulla Lantto, Petri Koivunen, Terhi Tapiainen, Marjo Renko
OBJECTIVE: To compare the effectiveness of tonsillectomy and the long-term outcome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in patients fulfilling the classic diagnostic criteria and in those with regularly recurring fever as the only symptom or with onset of symptoms after age 5 years or both. STUDY DESIGN: We reviewed the medical records of 3852 children who underwent tonsillectomy between 1990 and 2007 and identified 108 children who did so because of regularly recurring fevers...
September 27, 2016: Journal of Pediatrics
Kalpana Manthiram, Emily Nesbitt, Thomas Morgan, Kathryn M Edwards
OBJECTIVE: The goal of this study was to describe family history and inheritance patterns in patients with periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome. METHODS: We performed a case-control study to compare the family histories of patients with PFAPA recruited from Vanderbilt University Medical Center and matched healthy control subjects from a pediatric primary care practice in Nashville, Tennessee, by using a structured questionnaire...
September 2016: Pediatrics
Per Wekell, Anna Karlsson, Stefan Berg, Anders Fasth
UNLABELLED: There have been remarkable developments in the field of autoinflammatory diseases over the last 20 years. Research has led to definitions of new conditions, increased understanding of disease mechanisms and specific treatment. The polygenic autoinflammatory condition of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is the most common autoinflammatory disorder among children in many parts of the world. The clinical features often include clockwork regularity of episodes, prompt responses to corticosteroids and therapeutic effects of tonsillectomy, but the disease mechanisms are largely unknown...
October 2016: Acta Paediatrica
Federica Vanoni, Katerina Theodoropoulou, Michaël Hofer
The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment includes corticosteroids, which usually are efficacious in the management of fever episodes, colchicine, for the prophylaxis of febrile episodes, and other medication for which efficacy has not been proven so far. Tonsillectomy is an option for selected patients. Usually PFAPA syndrome resolves during adolescence, but there is increasing evidence that this condition may persist into adulthood...
June 27, 2016: Pediatric Rheumatology Online Journal
Nora S Ali, Julio C Sartori-Valinotti, Alison J Bruce
Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, the most common periodic disorder of childhood, presents with the cardinal symptoms of periodic fever, aphthous stomatitis, pharyngitis, and adenitis typically before age 5. This review presents the recent literature on PFAPA and summarizes key findings in the pathogenesis, evaluation, and treatment of the disease. Theories surrounding the pathogenesis of PFAPA include a faulty innate immunologic response in conjunction with dysregulated T-cell activation...
July 2016: Clinics in Dermatology
Maria Teresa R A Terreri, Wanderley Marques Bernardo, Claudio Arnaldo Len, Clovis Artur Almeida da Silva, Cristina Medeiros Ribeiro de Magalhães, Silvana B Sacchetti, Virgínia Paes Leme Ferriani, Daniela Gerent Petry Piotto, André de Souza Cavalcanti, Ana Júlia Pantoja de Moraes, Flavio Roberto Sztajnbok, Sheila Knupp Feitosa de Oliveira, Lucia Maria Arruda Campos, Marcia Bandeira, Flávia Patricia Sena Teixeira Santos, Claudia Saad Magalhães
OBJECTIVE: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation...
January 2016: Revista Brasileira de Reumatologia
Donato Rigante, Antonio Vitale, Marco Francesco Natale, Giuseppe Lopalco, Laura Andreozzi, Bruno Frediani, Francesca D'Errico, Florenzo Iannone, Luca Cantarini
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a mysterious disorder characterized by periodically recurrent fevers, oropharyngeal inflammation, and adenitis, which mainly affects children, though in very recent times, it has been also recognized in adulthood. We enrolled 115 unrelated pediatric and adult patients with history of periodic fevers who fulfilled the current diagnostic criteria for PFAPA syndrome in three Italian referral centers and highlighted differences between children and adults...
June 1, 2016: Clinical Rheumatology
Barbara Kraszewska-Głomba, Zofia Szymańska-Toczek, Leszek Szenborn
As no specific laboratory test has been identified, PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) remains a diagnosis of exclusion. We searched for a practical use of procalcitonin (PCT) and C-reactive protein (CRP) in distinguishing PFAPA attacks from acute bacterial and viral infections. Levels of PCT and CRP were measured in 38 patients with PFAPA and 81 children diagnosed with an acute bacterial (n=42) or viral (n=39) infection. Statistical analysis with the use of the C4...
2016: Bosnian Journal of Basic Medical Sciences
J P Windfuhr
BACKGROUND: One of the most significant clinical trials, demonstrating the efficacy of tonsillectomy (TE) for recurrent throat infection in severely affected children, was published in 1984. This systematic review was undertaken to compile various indications for TE as suggested in the literature after 1984 and to stratify the papers according to the current concept of Evidence-based Medicine. MATERIAL AND METHODS: A systematic Medline research was performed using the key word "tonsillectomy" in combination with different filters such as "systematic reviews", "meta-analysis", "English", "German" and "from 1984/01/01 to 2015/05/31"...
April 2016: Laryngo- Rhino- Otologie
Perrine Dusser, Véronique Hentgen, Bénédicte Neven, Isabelle Koné-Paut
INTRODUCTION: PFAPA syndrome is the most frequent periodic fever syndrome in non-Mediterranean patients. The pathogenesis is unclear and the treatment is purely symptomatic and not standardized. The aim of this study was to assess colchicine's efficacy as prophylactic treatment in PFAPA syndrome and to identify factors able to predict response to treatment. METHODS: We performed a retrospective, multicentric, cohort study of PFAPA patients under colchicine prophylaxis...
July 2016: Joint, Bone, Spine: Revue du Rhumatisme
Ezgi D Batu, Fehime Kara Eroğlu, Paul Tsoukas, Jonathan S Hausmann, Yelda Bilginer, Margaret A Kenna, Greg R Licameli, Robert C Fuhlbrigge, Seza Özen, Fatma Dedeoğlu
OBJECTIVE: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a periodic fever syndrome of childhood with an unknown etiology. Our aims were to compare the features between PFAPA patients from Turkey and the USA, and patients with and without MEFV variants, and to test the performance of the Eurofever criteria in excluding other autoinflammatory diseases. METHODS: Seventy-one children with PFAPA followed in Hacettepe University, Ankara, Turkey and 60 patients in Boston Children's Hospital, Boston, USA were enrolled...
April 5, 2016: Arthritis Care & Research
M V Tejesvi, M Uhari, T Tapiainen, A M Pirttilä, M Suokas, U Lantto, P Koivunen, M Renko
Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a childhood febrile syndrome of unknown origin that is often cured with tonsillectomy. We aimed to compare the bacterial microbiota of the tonsils removed from PFAPA patients with those of controls. We used next-generation sequencing technology to investigate the bacterial microbiota of the tonsils of 30 PFAPA patients and 24 controls. We found significant differences in the presence and relative abundance of many bacteria between PFAPA cases and controls...
June 2016: European Journal of Clinical Microbiology & Infectious Diseases
Katerina Theodoropoulou, Federica Vanoni, Michaël Hofer
PFAPA syndrome represents the most common cause of recurrent fever in children in European populations, and it is characterized by recurrent episodes of high fever, pharyngitis, cervical adenitis, and aphthous stomatitis. Many possible causative factors have been explored so far, including infectious agents, immunologic mechanisms and genetic predisposition, but the exact etiology remains unclear. Recent findings demonstrate a dysregulation of different components of innate immunity during PFAPA flares, such as monocytes, neutrophils, complement, and pro-inflammatory cytokines, especially IL-1β, suggesting an inflammasome-mediated innate immune system activation and supporting the hypothesis of an autoinflammatory disease...
April 2016: Current Rheumatology Reports
Soner S Kara, Duygu Kara, Ali Fettah
Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne disease with high mortality. Many disorders can mimic CCHF. It is important to recognize the condition and to perform differential diagnosis in endemic countries. Twenty-one children aged 18 years or less with a preliminary diagnosis of CCHF were retrospectively evaluated. Real-time PCR and a confirmatory indirect immunofluorescence assay for negative results were performed. The diagnoses determined that 9 patients had (42.9%) CCHF; 7 patients had (33.3%) viral upper respiratory tract infections (URTI); 2 patients had (9...
September 2016: Journal of Infection and Public Health
Erkan Demirkaya, Celal Saglam, Turker Turker, Isabelle Koné-Paut, Pat Woo, Matteo Doglio, Gayane Amaryan, Joost Frenkel, Yosef Uziel, Antonella Insalaco, Luca Cantarini, Michael Hofer, Sorina Boiu, Ali Duzova, Consuelo Modesto, Annette Bryant, Donato Rigante, Efimia Papadopoulou-Alataki, Severine Guillaume-Czitrom, Jasmine Kuemmerle-Deschner, Bénédicte Neven, Helen Lachmann, Alberto Martini, Nicolino Ruperto, Marco Gattorno, Seza Ozen
OBJECTIVE: Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean fever (FMF). METHODS: Pediatric patients with FMF from the Eurofever registry were used for the validation of the existing criteria. The other periodic fevers served as controls: mevalonate kinase deficiency (MKD), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA), and undefined periodic fever from the same registry...
January 2016: Journal of Rheumatology
Barbara Kraszewska-Głomba, Agnieszka Matkowska-Kocjan, Leszek Szenborn
BACKGROUND: PFAPA syndrome is a chronic disease that is characterized by recurrent episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Knowledge regarding the etiology of PFAPA is limited. OBJECTIVES: To provide up-to-date information considering etiology of PFAPA syndrome, by summarizing what has been explored and established in this area so far. MATERIALS AND METHODS: PubMed, Web of Science, and Scopus databases were searched for pertinent reports...
2015: Mediators of Inflammation
Mehmet H Celiksoy, Gonul Ogur, Elif Yaman, Ummet Abur, Semanur Fazla, Recep Sancak, Alisan Yildiran
BACKGROUND: The cause and pathophysiology of PFAPA syndrome is unknown. The aim of this study was to determine all MEFV gene variants relevant to familial Mediterranean fever in children with PFAPA syndrome. METHODS: All MEFV gene variants were analyzed in patients with PFAPA syndrome. All patients were evaluated using the Gaslini scoring system. Serum immunoglobulin levels were also determined upon admission. RESULTS: We evaluated 64 patients with PFAPA syndrome...
February 2016: Pediatric Allergy and Immunology
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